Bulkley B H, Weisfeldt M L, Hutchins G M
Circulation. 1977 Aug;56(2):292-8. doi: 10.1161/01.cir.56.2.292.
The specificity and significance of the asymmetric septal hypertrophy (ASH) and myocardial fiber disarray of idiopathic hypertrophic subaortic stenosis (IHSS) is uncertain. To examine this we studied 215 hearts, including normal embryos, fetuses, children, and adults; and hearts with congenital and acquired disease. Disproportionate septal thickening was present in all embryos and in some abnormal hearts, particularly those with severe right ventricular hypertrophy due to congenital malformations. Some myocardial fiber disarray was present in all hearts at the junctions of interventricular septum and ventricular free wall. In hearts with semilunar valve atresia with intact ventricular septums, and in the infundibulum of some with tetralogy of Fallot, however, extensive fiber disarray was present. Thus, ASH occurs in the normal developing heart and in some malformed hearts with RVH; marked muscle fiber disarray may occur in certain congenital lesions with abnormal systolic contraction. Neither morphologic finding independently or in combination is pathognomonic of idiopathic hypertrophic subaortic stenosis.
特发性肥厚性主动脉瓣下狭窄（IHSS）的不对称性室间隔肥厚（ASH）和心肌纤维排列紊乱的特异性及意义尚不确定。为研究此问题，我们研究了215颗心脏，包括正常胚胎、胎儿、儿童及成人的心脏，以及患有先天性和后天性疾病的心脏。所有胚胎及部分异常心脏，尤其是那些因先天性畸形导致严重右心室肥厚的心脏，均存在不成比例的室间隔增厚。在室间隔与心室游离壁交界处的所有心脏中，均存在一些心肌纤维排列紊乱。然而，在室间隔完整的半月瓣闭锁的心脏以及部分法洛四联症患者的漏斗部，存在广泛的纤维排列紊乱。因此，ASH见于正常发育的心脏以及一些伴有右心室肥厚的畸形心脏；在某些收缩期收缩异常的先天性病变中，可能出现明显的肌纤维排列紊乱。这两种形态学表现单独或联合出现，均非特发性肥厚性主动脉瓣下狭窄的特征性表现。
